신경모세포종

강형진,유경하,신희영,안효섭,Kang, Hyoung-Jin,Ryu, Kyung-Ha,Shin, Hee-Young,Ahn, Hyo-Seop 대한소아외과학회 2008 소아외과 Vol.14 No.1

Neuroblastoma arises from the primitive neural crest cells, and is a common malignancy in childhood. The clinical features are characterized by biological heterogeneity. Neuronal degeneration and differentiation occur in some patients. However treatment in the high risk group accounting for approximately half, has not been satisfactory despite a multimodal approach. Therefore, effective treatment is determined by the risk group of prognostic factors, such as age at diagnosis, stage of disease, pathological finding and N-myc amplification. Neuroblastoma can be diagnosed prenatally, which suggests its origin during the normal embryogenesis. Recent knowledge of molecular biology, such as Trk genes, and the concept of cancer stem cells have given us some improved understanding on this disease. Currently, targeted therapies based on the molecular biology of neuroblastoma are under investigation and increasing survival rate and decreasing late complications could be appreciated.

척수내 유피낭종 환아에서 발생한 화농성 척수공동증 1례

강형진(Hyoung Jin Kang),김기중(Ki Joong Kim),황승용(Yong Seung Hwang),김인원(In-One Kim),조병규(Byung Keu Cho) 대한소아신경학회 1995 대한소아신경학회지 Vol.3 No.1

저자들은 5개월된 남아에서 유피동과 척수내 유피낭종이 척수강에 연결되어 예후가 나쁜 급성, 다발성의 척수내 농양, 즉 화농성 척수공동증으로 나타난 증례를 자기 공명영상으로 진단하고 항생제와 여러 차례의 배농으로 신경학적 호전을 보인 후에 수술로 원인을 제거하여 생존한 증례를 국내에서는 처음으로 경험하였기에 보고하는 바이다. A 5 month-old male infant presented with high fever and progressive motor weakness of both lower extremities. He had skin dimpling on the back. Spine MRI reaveled that he had multiple intramedullary abscesses which are connected with hydrosyringomyelia, intramedullary dermoid cyst and dermal sinus tract. Initially he was treated with antibiotics and frequent spinal tapping for one month. Consequently his neurologic signs began to improve, so the operation was done. It is considered that he might be the first case who survived with frequent spinal tapping before surgery.

소아 Anthracycline 심독성의 추적 관찰

권혁주,송영환,강수정,강형진,최형수,배은정,신희영,노정일,윤용수,안효섭,Kwon, Hyok Joo,Song, Young Hwan,Kang, Soo Jung,Kang, Hyoung Jin,Choi, Hyoung Soo,Bae, Eun Jung,Shin, Hee Young,Noh, Chung Il,Yun, Yong Soo,Ahn, Hyo Seop 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.3

Purpose : We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. Methods : A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two groups, mild and severe cardiotoxicity, according to the FS. We followed up survivors with cardiotoxicity, and checked their present cardiac function by physical activity, echocardiography, electrocardiography(EKG) and chest X-ray. Results : Of the 154 children treated with anthracyclines, forty(26.0%) were diagnosed as cardiotoxicity. The incidence of cardiotoxicity increased in exponential fashion with increases in the cumulative dose of anthracyclines. There was minimal increase of incidence until a dose of $300mg/m^2$ after which the incidence increased rapidly. After mean $3.8{\pm}1.8year$ follow-up of 23 survivors with cardiotoxicity, FS increased significantly. EKG and chest X-rays were not helpful for the diagnosis of cardiotoxicity because of their low sensitivity and specificity. Conclusion : Although convenient, non-invasive and inexpensive, EKG and chest X-rays were not helpful for the follow-up of anthracycline cardiotoxicity. Almost all survivors with anthracycline cardiotoxicity have improved in both physical activity and echocardiographic findings after discontinuation of anthracyclines. 목 적 : 항암치료 중 anthracycline을 사용한 환아들에 대한 후향적 조사를 통해 anthracycline의 축적량과 anthracycline 심독성과의 관계를 알고자 했고 심독성 환아들에 대한 추적 조사를 통해 anthracycline 심독성 환아의 예후를 알고자 하였다. 방 법 : 1995년 1월부터 2000년 12월까지 서울대학교병원 소아과에서 anthracycline을 포함한 항암치료를 시작한 환아를 대상으로 과거 anthracycline 축적량과 심장 초음파 검사 소견, 심독성 발생 유무 등을 조사하였다. 심독성은 좌심실 수축분율이 26% 이하로 감소한 경우로 정하였고 이들을 다시 좌심실 수축분율이 20%가 넘는 경증 심독성과 20% 미만인 중증 심독성으로 분류하였다. Anthracycline 심독성이 발생한 환아들에게 운동 능력과 심장 초음파, 심전도 검사, 흉부 방사선 촬영 등의 추적 검사를 시행하였다. 결 과 : 총 조사대상 환아는 154명이었다. 이 중에서 40명(26.0%)의 anthracycline 심독성이 발생하였으며 경증 심독성이 27명(17.5%), 중증 심독성이 13명(8.4%)이었다. 심독성의 발생률은 anthracycline의 축적량이 증가함에 따라 서서히 증가하다가 축적량이 $300mg/m^2$ 이상부터 갑자기 지수적으로 증가하는 양상을 보였다. 심독성 환아 40명 중 12명이 추적조사 이전에 사망하였고 생존한 환아 중 23명에 대해 추적검사를 시행하였다. Anthracycline의 사용을 중단한 후 평균 $3.6{\pm}1.8$년의 추적검사 결과 심독성 환아들의 좌심실 수축분율은 유의하게 증가하였다(P<0.01). 흉부 방사선 촬영이나 심전도 검사는 심독성의 진단을 위해 민감도와 특이도가 매우 낮은 검사였다. 결 론 : 일반적으로 anthracycline 심독성 환아의 추적검사로 시행하는 심전도 검사나 흉부 방사선 촬영은 심독성의 추적 조사에 도움이 되지 않는다. 심독성 환아들은 일단 생존하여 항암치료를 마치고 약 2-5년 정도 경과하면 심부전 증상이나 심장초음파 검사상 대부분 호전을 보인다.

목표주차위치 설정방법

정호기(Ho Gi Jung),강형진(Hyoung Jin Kang),김재희(Jaihie Kim) 한국자동차공학회 2008 한국자동차공학회 춘 추계 학술대회 논문집 Vol.- No.-

다차량 주행 상황에서의 자동주행/충돌회피 제어 알고리즘 설계

문승욱(Seungwuk Moon),강형진(Hyoung-Jin Kang),윤팔주(Paljoo Yoon),이경수(Kyongsu Yi) 한국자동차공학회 2008 한국자동차공학회 춘 추계 학술대회 논문집 Vol.- No.-

최근 5년(2006-2010)간 소아 혈액 종양 환자에서 발생한 균혈증의 원인균 및 임상 양상: 단일기관 연구

강지은,석준영,윤기욱,강형진,최은화,박경덕,신희영,이환종,안효섭,Kang, Ji Eun,Seok, Joon Young,Yun, Ki Wook,Kang, Hyoung Jin,Choi, Eun Hwa,Park, Kyung Duk,Shin, Hee Young,Lee, Hoan Jong,Ahn, Hyo Seop 대한소아감염학회 2012 Pediatric Infection and Vaccine Vol.19 No.3

목 적 : 소아 종양 환자의 균혈증의 원인균과 위험 인자에 대한 지속적인 감시 및 조사를 통해 적절한 치료와 생존율 향상에 기여하고자, 2006년부터 2010년의 최근 5년간 서울대학교 어린이 병원 소아 종양 환자에게 발생했던 균혈증의 원인균 분포와 이들의 항생제 감수성을 분석하여 항생제 선택에 실제적인 도움을 얻고자 하였다. 방 법 : 2006년 1월부터 2010년 12월까지 5년간 서울대학교 어린이병원에서 입원 치료를 받은 소아 종양환자들에게 발생한 균혈증에 대하여, 의무기록을 후향적으로 분석하였다. 의무기록 분석 시, 선행 질환 및 호중구 수치, 치료 경과 등을 조사하였고, 호중구 감소증, 중심 정맥관 등 균혈증의 예후 인자에 대한 분석을 함께 시행하였다. 결 과 : 총 176명의 소아 종양 환자에게 226례의 균혈증이 발생하였고, 246균주가 분리되었다. 이 중 그람 음성균, 그람 양성균, 진균이 각각 63.4%, 34.6%, 2.0%이었다. 그람 음성균 중에는 Klebsiella species, E. coli, 그람 양성균 중에는 coagulase-negative staphylococci, S. aureus 등이 높은 비율로 분리되었다. 그람 양성균의 penicillin, oxacillin, vanconycmin 내성률은 각각 85.7%, 65.9%, 9.5%이었으며, 그람 음성균의 cefotaxime, piperacillin/tazobactam, imipenem, gentamicin, amikacin 내성률은 각각 37.2%, 17.1%, 6.2%, 32.2%, 13.7%이었다. 전체 환자에서 치사율은 12.7%였다. 그람 음성균 균혈증의 쇼크 발생률이 그람 양성균 균혈증보다 유의하게 높았고(48.4% vs. 11.9%, P<0.01), 치사율 역시 그람 음성균이 더 높았다(12.1% vs. 3.0%, P=0.03). 쇼크 발생률은 호중구 감소증을 동반한 환자군에서 호중구 감소증을 동반하지 않은 환자군에서보다 유의하게 높았다(39.6% vs. 22.0%, P=0.04). 결 론 : 그람 음성균이 소아 종양 환자의 균혈증의 흔한 원인균이며, 불량한 예후와도 관련성이 높은 점들은 기존 연구결과들과 일치하였다. 향후 원인 균주의 분포와 항생제 내성률의 변화에 대한 지속적인 모니터링이 필요할 것으로 생각된다. Purpose : This study was performed to identify the etiologic agents and antimicrobial susceptibility patterns of organisms responsible for bloodstream infections in pediatric cancer patients for guidance in empiric antimicrobial therapy. Methods : A 5-year retrospective study of pediatric hemato-oncologic patients with bacteremia in Seoul National University Children's Hospital, from 2006 to 2010 was conducted. Results : A total of 246 pathogens were isolated, of which 63.4% (n=156) were gram-negative, bacteria 34.6% (n=85) were gram-positive bacteria, and 2.0% (n=5) were fungi. The most common pathogens were Klebsiella spp. (n=61, 24.8%) followed by Escherichia coli (n=31, 12.6%), coagulase-negative staphylococci (n=23, 9.3%), and Staphylococcus aureus (n=22, 8.9 %). Resistance rates of gram-positive bacteria to penicillin, oxacillin, and vancomycin were 85.7%, 65.9%, and 9.5%, respectively. Resistance rates of gram-negative bacteria to cefotaxime, piperacillin/tazobactam, imipenem, gentamicin, and amikacin were 37.2%, 17.1%, 6.2%, 32.2%, and 13.7%, respectively. Overall fatality rate was 12.7%. Gram-negative bacteremia was more often associated with shock (48.4% vs. 11.9%, P<0.01) and had higher fatality rate than gram-positive bacteremia (12.1% vs. 3.0%, P=0.03). Neutropenic patients were more often associated with shock than non-neutropenic patients (39.6 % vs. 22.0%, P=0.04). Conclusion : This study revealed that gram-negative bacteria were still dominant organisms of bloodstream infections in children with hemato-oncologic diseases, and patients with gram-negative bacteremia showed fatal course more frequently than those with gram-positive bacteremia.

Voriconazole Therapeutic Drug Monitoring is Necessary for Children with Invasive Fungal Infection

강현미,강수영,조은영,유경상,이지원,강형진,박경덕,신희영,안효섭,이현주,최은화,이환종,Kang, Hyun Mi,Kang, Soo Young,Cho, Eun Young,Yu, Kyung-Sang,Lee, Ji Won,Kang, Hyoung Jin,Park, Kyung Duk,Shin, Hee Young,Ahn, Hyo Seop,Lee, Hyunju,Choi, The Korean Society of Pediatric Infectious Disease 2014 Pediatric Infection and Vaccine Vol.21 No.1

목적: 본 연구는 소아 환자들에서 voriconazole 치료적 약물 농도 모니터링의 임상적 의의를 분석하고자 하였다. 방법: 2010년 7월부터 2012년 6월까지 서울대학교병원에 입원한 18세 이하의 소아 환자들 중, 침습성 진균감염증에 대해 voriconazole 치료를 받은 증례를 후향적 의무기록 분석을 통해 분석하였다. 본 연구에 포함된 총 28명의 환자 중 14명이 약물 농도 모니터링을 받았으며, 143개의 혈중 농도 측정 값을 분석하였다. 모든 환자들에게서 치료 효과 및 독성 증상 발현 여부를 파악하였다. 결과: 143개의 혈중 농도 측정 값 중 53.1%에서 치료적 범위(1.0-5.5 mg/L) 내에 들었고, 같은 용법으로 치료받았더라도 높은 혈중 농도 변동성(high variability)을 보였다. 약물 농도 모니터링을 받았던 군(TDM 군)과 받지 않았던 군(non-TDM 군)에서 각각 14명 중 9명(64.3%)이 독성 증상을 나타냈는데, TDM 군에서 신경학적 증상(n=2, 14.3%) 및 간기능 장애(n=8, 57.1%)는 높은 voriconazole 혈중 농도(>5.5 mg/L)를 보인 환자들에게서 나타났다. 반면, 시각 장애는 혈중 농도가 치료적 범위 내에 있을 때 발현하였다(1.18 mg/L, 3.9 mg/L). TDM 군에서 non-TDM 군에 비하여 독성 증상으로 인하여 약물을 중단했던 빈도가 낮았다(0.0% vs. 18.2%, P =0.481). 치료 시작 6주 후 치료 효과를 분석해본 결과 TDM 군의 57.2%에서 치료에 대한 반응을 보였으나, non-TDM 군에서는 14.3%에서 치료 반응을 보였다(P =0.055). 최종 치료효과 분석에서는 TDM 군의 21.4%에서 치료 반응을 보였으나, non-TDM 군의 14.3%에서 치료 반응을 보였다(P =0.664). TDM 군에서 치료 시작 첫 6주 동안 혈중 약물 농도를 분석했을 때 67.0% 이상에서 치료적 범위 내에 들었으나, 치료 기간 전체를 봤을 때에는 45.5%에서 치료적 범위 내에 들었다. 결론: 소아에서 voriconazole 사용 시 치료적 약물 농도 모니터링을 통하여 치료 목표를 효과적으로 달성하고, 독성이 나타나는 것을 예방할 수 있다. Purpose: To determine the clinical significance of voriconazole therapeutic drug monitoring (TDM) in the pediatric population. Methods: Twenty-eight patients with invasive fungal infections administered with voriconazole from July 2010 to June 2012 were investigated retrospectively. Fourteen received TDM, and 143 trough concentrations were analyzed. All 28 patients were assessed for adverse events and treatment response six weeks into treatment, and at the end. Results: Out of 143 samples, 53.1% were within therapeutic range (1.0-5.5 mg/L). Patients administered with the same loading (6 mg/kg/dose) and maintenance (4 mg/kg/dose) dosages prior to initial TDM showed highly variable drug levels. Adverse events occurred in 9 of 14 patients (64.3%) in both the TDM and non-TDM group. In the TDM group, voriconazole-related encephalopathy (n=2, 14.3%) and aspartate aminotransferase (AST) or alanine aminotransferase (ALT) elevation (n=8, 57.1 %) occurred with serum levels in the toxic range (>5.5 mg/L), whereas blurred-vision (n=2, 14.3%) occurred within the therapeutic range (1.18 mg/L and 3.9 mg/L). The frequency of voriconazole discontinuation due to adverse events was lower in the TDM group (0.0% vs. 18.2%, P =0.481). Overall, 57.2% of the patients in the TDM group versus 14.3% in the non-TDM group showed clinical response after 6 weeks (P =0.055), whereas 21.4% in the TDM group versus 14.3% in the non-TDM group showed response at final outcome (P =0.664). In the TDM group, >67.0% of the serum levels were within therapeutic range for the first 6 weeks; however 45.5% were within therapeutic range for the entire duration. Conclusion: Routine TDM is recommended for optimizing the therapeutic effects of voriconazole.

신생아기 신경모세포종의 임상적 고찰: 산전 진단군과 산후 진단군의 비교

박훤함,김수홍,정성은,이성철,박귀원,이지원,강형진,신희영,백해운,김현영,Park, Hwon Ham,Kim, Soo-Hong,Jung, Sung-Eun,Lee, Seong-Cheol,Park, Kwi-Won,Lee, Ji Won,Kang, Hyoung Jin,Shin, Hee Young,Baek, Hae Woon,Kim, Hyun-Young 대한소아외과학회 2014 소아외과 Vol.20 No.2

Purpose: Neonatal neuroblastoma (NBL) is the most common malignant tumor in neonates, but there have been few studies about it. The purpose of this study was to investigate the clinical features of NBL and to compare prenatal and postnatal diagnosed groups. Methods: Nineteen patients who were diagnosed with NBL prenatally or within 28 days after birth from February 1986 to February 2013 in Seoul National University Hospital were enrolled in the study. The patients were categorized according to the International Neuroblastoma Staging System (INSS) and Children's Oncology Group (COG). Retrospective medical-record reviews were performed on these patients. The operative date, complication, pathological stage, and overall survival of the prenatally diagnosed group and the postpartum diagnosed group were compared. Results: Tumor was detected via prenatal ultrasonography in 8 patients (42.1%), and 11 patients (57.9%) were diagnosed within 28 days after birth. Based on INSS, the patients were divided into the stage I (n=8), stage II (n=1), stage III (n=3), stage IV (n=4), and stage IVs (n=3) groups, respectively. Based on COG, on the other hand, the patients were divided into the low-risk (n=8), intermediate-risk (n=8), and high-risk (n=3) groups. The postoperative complication rate was 29%. One patient died from complications from chemotherapy. The other 18 patients' mean follow-up period was 77.7 months. The differences between the postoperative complication rate, proportion of early-stage tumor, and overall survival of the prenatal and postnatal groups were not statistically significant (p=0.446, p=0.607, p=0.414). Conclusion: NBL showed favorable outcomes but relatively higher postoperative complications. There seem to be no significant statistical differences in the postoperative complications, proportion of early-stage tumor, and overall survival between the prenatally diagnosed group and the postpartum diagnosed group.

급성 신부전을 동반한 발작 야간 혈색소뇨증 2례

류정민,안요한,이소희,최현진,이범희,강희경,강형진,신희영,하일수,정해일,안효섭,최용,Ryu, Jung-Min,Ahn, Yo-Han,Lee, So-Hee,Choi, Hyun-Jin,Lee, Beom-Hee,Kang, Hee-Gyung,Kang, Hyoung-Jin,Shin, Hee-Young,Ha, Il-Soo,Cheong, Hae-Il,Ahn, Hyo-Se 대한소아신장학회 2008 Childhood kidney diseases Vol.12 No.2

Paroxysmal nocturnal hemoglobinuria(PNH) is an acquired hematologic disorder characterized by intravascular hemolysis, nocturnal hemoglobinuria, thrombotic events and bone marrow failure. It rarely occurs in children and can be complicated by acute renal failure(ARF). Here, we present two cases of ARF complicating PNH which has not been reported yet in Korean children. We suggest that PNH should be considered in differential diagnosis of ARF in children associated with intravascular hemolysis. PNH는 혈관내 용혈, 야간 혈색소뇨, 혈전증, 골수부전을 특징으로 하는 후천적 질환이며 소아에서는 매우 드물게 발생한다. 급성 신부전은 PNH의 합병증으로 알려져 있지만 우리나라 소아에서의 보고는 아직 없었다. 저자들은 소아에서 PNH로 인한 급성 신부전을 경험하였기에 이를 보고하며 용혈을 동반한 소아의 급성 신부전의 감별 진단에서 PNH가 포함되어야 한다고 생각한다.

난치성 편측 흉수와 불응성 혈소판 감소증으로 나타난 흉강 내 혈관내피종 1예

김효원 ( Hyo Won Kim ),최윤정 ( Yun Jung Choi ),홍경택 ( Kyung Taek Hong ),강형진 ( Hyoung Jin Kang ),박경덕 ( Kyung Duk Park ),김세희 ( Sehui Kim ),최영훈 ( Young Hoon Choi ),김우선 ( Woo-sun Kim ),서동인 ( Dong In Suh ) 대한천식알레르기학회(구 대한알레르기학회) 2016 Allergy Asthma & Respiratory Disease Vol.4 No.6

A variety of diseases are associated with the development of unilateral pleural effusion. Although unilateral pleural effusion is common, refractory unilateral pleural effusion is rare. It is important to make an accurate diagnosis using proper diagnostic tools. Thrombocytopenia is one of the rare conditions occurring from various diseases such as severe infection or autoimmune diseases. It can be life-threatening if accurate diagnosis and treatment are delayed and be a clue to accurate diagnosis in differential diagnosis from refractory pleural effusion. Kasabach-Merrit syndrome (KMS) is often accompanied by extensive vascular tumors and characterized by consumptive coagulopathy with profound thrombocytopenia. It is also important to have a high index of suspicion for the diagnosis. We report a case of KMS in a 2-month-old female infant with a vascular tumor on her left intrathoracic cage, who had presented refractory unilateral pleural effusion and thrombocytopenia. Initially, the patient was diagnosed as having complications of severe infection, and a chest tube was inserted for aggressive treatment. However, her unilateral pleural effusion persisted, and thrombocytopenia and hypofibrinogenemia were refractory. Chest imaging revealed an infiltrating large vascular tumor involving the cardiac border, diaphragm, and chest wall. The patient`s unilateral pleural effusion was misidentified as an infectious condition at the initial stage. As a result of the ultrasonography-guided biopsy, it was revealed to be Kaposiform hemangioendothelioma. The patient was cured after treatment for KMS. (Allergy Asthma Respir Dis 2016:4:453-457)